Thursday, July 31, 2014

Neurosurgery post-surgery Appt. Looking at IT/Dr.Dickson, talking to Plastic Surgery/UW

A bit earlier I was talking to one of the former D6-4 (Neurosurgery) fl Nurses on fb about a book I gave her and we got to talking about Dr.Bragg/today's appt.
I said to her jokingly in talking about today's appt and how we had a tentative plan how grateful I am still that Dr.Bragg keeps trying and made the comment 'She's a good one Mr.Grinch'. I think the real saying is "She's a mean one Mr.Grinch" but really I con't to be amazed how willing Dr.Bragg is to con't to try to help make my shunts work better and my symptoms overall better. AND that she sees me mostly at good pts of the day but she still believes me! To be honest I am continually (but so grateful) amazed!

zzz I brought up to Dr.Bragg about how the 2 drs I had talked weren't real confident a cranial vault expansion (thinning the skull bone, create a bit more room for the brain to expand) would help w our issues w seeming not enough fluid being drained/headaches/keeping the shunts working. I also brought up how these same 2 drs have suggested to me I ask again about trying to do IT Aldurazyme and I wondered if she thought this might be something we could try to get approved at UW as an outside study?
Dr.Bragg made the comment when I asked her that she didn't know a lot about IT as I am her only MPS pt and that we would have to try to get an IRB (internal review board) approval at UW but she was open to talking to Dr.Dickson and I should send her the contact info.
Dr.D and Dr.Bragg had talked a year or more ago about the actual IT Study in CA but I wouldn't likely qualify, most especially due to my shunts, repeated spine surgeries and unlikely ability to have LP's which is their means of injecting the Aldurayzme intrathecally.
When I was originally in Dr.Dickson's first Cord Compression IT Study we had had to get an IRB exception to do the injections in to the cisterna magna (base of the skull) due to my then LP Shunt.
Dr.Bragg made the comment we would have to likely insert a reservoir to inject the drug via likely. I think per these 2 drs their recommendation had been to me they thought a way to virtually turn the shunts off for a few hrs (or turn as high as they can, to drain as little as shunts can) would be a good idea (to allow the drug to bathe as long as possible as Aldurazyme doesn't otherwise cross the BBB during the weekly IV (Port-a-cath) Enzyme Repl. Infusions I get.
Dr.Bragg is going to talk to Dr.Dickson (Patti replied almost immediately to my email) and see what Dr.Dickson (Patti) suggests as far as going about trying to get approval and if approved how to set it up.

Additionally Dr.Bragg and I talked about the skull bone thinning (cranial vault expansion) and agreed we'll keep looking in to this, she'll talk to her Peds Plastic Surgery colleague who returns next wk. and see what that dr (Dr.Mount) thinks.. ie does Dr.Mount think w the underlying MPS it could help, is it feasible, etc. If need be we'll meet the 3 of us and discuss that. For now it's sort of our least desired option which I am more than ok with!

I could tell Dr.Bragg really wished she had some way to help immediately today. She asked if there was anything I wanted (thought) she could do to help? She had the shunt re-programmer with her but we opted to leave things are as at least while very imperfect I can take Zofran and it helps w nausea. If it gets very bad (some nights) I can take a exceedingly small amount of the valium to get back to sleep (this is like a last resort to me).
Via pumping the VP Shunt reservoir the refill time was ok. Our suspicion is always that this is the shunt that works the least effectively more due to overdrainage (even though I do best draining a lot) and thus the ventricles collapse, sucking brain debris in to the catheters. Our wish is really to find a way to make the shunts work tip top.
We discussed the TPL Shunt fluid seeming to accumulate but I didn't think to ask her about the strong discomfort that keeps occurring (and PT is really trying to help) down the back of my L butt and L/hamstring area.
Honestly I am impressed with myself that I at least remembered to ask her all the things I did though (!!) so I think it was a reasonably successful and accomplished appt!
One thing I did ask her was why she might think I (and others I've talked to have commented the same thing) would notice a greater difference in symptoms when we do a full shunt replacement vs when we do a partial revision (just a small part of the shunt)? She wasn't really sure though one thing she thought was possible was that with a full revision you are under anesthesia for longer and this impacts CSF pressure longer, to a greater degree thus you may feel like you feel better (or something like that).
I honestly am not sure I think that is true, nor has it been my experience but maybe for some it is. In my own case I think anesthesia no matter what seems to impact my body for days but not necessarily in a positive way.
In my own case though I think that has a lot to do with the endocrine hormone issues, the affect anesthesia and pressure has on these hormones and although it makes a significant, positive difference (the steroid use for cortisol stress dosing) my body still seems to go through some stress response. I might be wrong though.
She also commented to the Resident and Student that where with her when we where talking about the possibility of IT, about her talking to the Plastic Surgeon next wk and about the shunts in general that our best that she has seen cont's to be with either the External Drains (EVD's) or when we had the external (but under the skin) CSF fluid leaks. I would have to agree with her on those thoughts! While having the 2nd shunt as I've written about before atleast helps b/c there is always atleast some pressure being drained having the 2 shunts is no the same as how good I felt w the CSF leak or the EVD's!
We did discus the possibility of adding the siphon guard further down on the VP Shunt to prevent over-drainage which in turn leads to the shunts occluding. The thought behind this is preventing over drainage would in theory or they've been able to establish help avoid the pressure having to build significantly in order to force the catheters back open after over-draining.
Her (and my) concern with this is I do best the more I can drain and anti-siphon guard valves allow only limited amounts of flow and I believe are positional. It's something we've only semi-ruled out I think but also not entirely leaning towards as out best option.

After the appt I went up and visited some friends who are in-patient. Their little girl has a lot of shunt issues like I do though sees Dr.Bragg's partner.
Shannon often comes to visit me while I am in-pt and I genuinely enjoy talking to her and her husband. I wish I honestly I knew better how to help their little girl feel better though (but I am sure they do to!
You would think I guess given we have the same shunt problems it'd be so easy to know but it's not! I am really glad I could visit though.
Shannon is who will often access my Port these days when surgery is on a Monday or if I can't get to Milw to get it re-accessed (Noodle, her little girl has a Port and Shannon is a Lab Tech).
Honestly, I kind of think she and her hubby are awesome (fairly close in age to me), all of their 4 kids are adopted with 2 having special needs (their little boy is similar needs to Zan and also high functioning though I don't know if he is technically on the spectrum) while Noodle has the shunt and I believe CP. They are also in the midst of finalizing adoption of another little girl from Krygzstan (sp) and Shannon flies there in less than 1 1/2 wks for the Court appearance!

Thanks for stopping by,
If you believe in prayer, please pray we are able to sort out the above things in a not to lengthy amount of time and also find a solution to the shunt issues!
I am so very grateful to have Dr.Bragg on my 'Team' and that she never seems to give up!

Saturday, July 19, 2014

Updated: TPL Shunt site better post-surgery. VP Shunt iffy? Discharged Fri (18th)

Sorry for the delay in writing an update; I am home (Fri) which if nothing else is nice! I hate writing these updates the most as I can imagine it must get tiring for those who read this to continually read about problems. I promise I would love to write all good updates for once to! =) To boot uncertainty makes me apprehensive (atleast in regard to the shunts) and it is a bit daunting (scary?) not having a plan.
I do atleast feel a bit better of the whole situation today than I did over the past few days. I do just want to feel better though!

Due to what I am more than certain is a valve issue and on-going, slowly worsening symptoms (VP Shunt) Dr.Bragg opted to do a shunt tap Fri morning which showed the valve is working but seemed was slow CSF flow (not entirely sure) when she was pulling off fluid. In the past this has been indicative of partial or intermittent shunt blockage; there have been numerous times we've just ended up revising that portion of the shunt (the valve).
She was able to remove with the shunt tap I think she said about 2cm (or 2cc's?) of CSF for which I can only best describe as it felt literally like a heavy cloud (cobwebs?) is partially lifted from my head. A very odd but not terrible feeling.
It's very clear the symptoms at night/morning especially had gradually gotten worse over the past few weeks. My symptoms after this shunt tap where virtually back to 'normal' by Sat morning, meaning I did not feel as good as I had headache wise after we did the shunt tan and took off some fluid.
I trust that there is a reason for not being certain what to do next but admittedly feeling like crap a good percent of the day and "just doing what I do at home" to get through it sucks (though I am happier to be here than to be in-pt). Atleast at home I control my day to day. It would be sooo amazing to just wake up and not have to feel aweful, literally feeling like your waiting as the day slowly gets better!

Even with some relief from the shunt tap (the first 24 hrs anyways) my vision is screwed up (fuzzy I guess, worse with reading) and head a day later feels like a lump of coal sitting on it just initially was not as bad as pre-tap! The symptoms do occur intermittently and worse at certain points of the day.
I think with the CSF that was pulled of with the tap she was planning to send off the CSF sample for testing though it is unlikely there would be an infection (no symptoms) and she I guess just wanted to be cautious, rule things out. I am ok with that, glad I guess she wanted to try and cover bases.
The bad thing with our relieving some pressure Fri morning is of course the headaches return and sometimes that is worse than not getting any relief till we figure out a actual plan! You can almost taste feeling better, longer with times like this where some pressure is relieved! Still I am glad even if it wasn't perfect relief to have felt a little better.
BR> We do know the TPL Shunt is working (a very good thing!!) based off flow during the TPL Shunt revision surgery on Monday. Thankfully the pinching/irritation symptoms from the TPL Shunt rubbing is also improved following surgery which is AWESOME! One of the incision areas from Monday's surgery surprisingly is still sore. This was done via laparoscopic (tiny) incisions but perhaps it's the location that causes the discomfort. ATLEAST the pinching/discomfort along the TPL Shunt is virtually (if not completely!) gone post-surgery!

As far as what is our plan, I believe Dr.Bragg when she says there are options but it doesn't make the time while waiting to figure out what the next step is any easier. Even Dr.Bragg seems to be struggling with what to do but better she takes her time to figure out a plan I guess. One possible thing she has circled back to is doing the cranial vault expansion surgery (where they go in a literally shave down some of the skull bone - if it sounds aweful to you believe me it sounds AWEFUL to me to!
The Peds Plastic Surgeon that helps Dr.Bragg w skull expansion surgeries is out till the 1st of August and I think it would likely be something we'd take some time to investigate (If it would work w the underlying MPS). I am more than leery of it!
In my case I think she said they would only (only!) need to shave down the skull bone and would not she believes need to reshape the actual skull. Does. Not. Sound. Awesome! I really don't know any other details about this but I think she is concerned we keep having to revise the shunts over and over and over again with limited success. It's to bad we couldn't figure out a way to replicate the TPL Shunt function (which we know works best out of the 2 shunts) in the VP Shunt!?
As far as recovery it seems if I am remembering Dr.Bragg right that is about 2 wks post-op in patient and they use plates and screws to hold the skull back together. Can you imagine? UGH!
I've talked to a couple drs in the MPS community about the potential skull bone thinning and both feel this may not work and little precedent (if any) for it's use in MPS. The one dr must have quickly done some research to see if she could find any published data on it's use and didn't have a lot of success.
The other dr who has a ton of experience (dozen of years) with MPS I pts brought back up the potential to look in to IT Aldurazyme via the shunt reservoir which is something I wish we could work out.
I guess i'll ask Dr.Bragg if it might be something we could look in to again and would she be willing to talk to Dr.Dickson (They had talked one other time)? I know there is 1 other MPS pt (not a shunt pt though) whose local dr was able to work w Dr.Dickson to get the IT (Aldurazyme) approved for out of study use.
Dr.Dickson has said she would be willing to try and help Dr.Bragg get it approved at UW if she wanted/was interested. I feel like we should try to look in to everything.. The idea behind IT in to a shunt is that they temporarily re-program the shunts to a 'virtual off' setting and inject the aldurazyme via a reservoir in-line w the shunt catheter.
Dr.Kakkis feels there would have to be a one way valve (not sure if the Codman Hakim is a one way valve). The thought is that Aldurazyme given directly in to a shunt reservoir may help clear our GAG storage shed from the ependymal
Dr.Kakkis also wondered if some type of reservoir could be used to flush the shunt catheter out with forced back pressure every once in awhile.. I am curious about this and something else to fwd to Dr.Bragg. I do know the catheters clogging is not unique to MPS pts but am unsure if it is more common with our disorder or a different unique? It seems it might not be but again feel like we have to look at everything..
Something I have wondered but didn't think of till after I talked to Dr.Bragg is I wonder can Hydrocephalus pts who do well draining a lot of fluid ever get shunted without an actual valve in place (valves control flow) on their shunt? Something to keep in mind to ask.. I would imagine a valve is needed but is worth asking.

I know as far as ideas she was going to talk to her Codman Rep about a siphon guard valve (controls amount of flow when person stands up) but she (and I for that matter) aren't sure that is a good option as I do better the more CSF I can drain.
I am doubtful she would decide to go that route and as much as I want to feel better I guess I am ok her taking her time trying to figure out a better option.

I guess I need to do better, trust whatever she decides and take these next few wks a day at a time.. I will do what I do well and that is enjoy the moments that are good while just living with the times that are not.
These periods in-between feeling good are definitely when my faith and trust are tested!

I'll update again when there is something to update,
Thanks for stopping by,
Ps Sorry I realize this update is fairly scattered and not all that well written!